With respect to ATP8B1, ABCB11 and ABCB4, it seems increasingly likely that pathogenic changes in these genes may be implicated in a whole spectrum of disease, ranging from the severe progressive cholestatic disease seen in PFIC to intermittent forms such as benign recurrent intrahepatic cholestasis (BRIC), drug-induced cholestasis (DIC), intrahepatic cholestasis of pregnancy (ICP) and LPAC. The gene discussed is ABCB11; the disease is progressive familial intrahepatic cholestasis.